Background: Synovial sarcoma is an aggressive but chemosensitive soft-tissue tumor. We retrospectively analyzed the efficacy of perioperative chemotherapy for synovial sarcoma with data from the nationwide database, Bone and Soft Tissue Tumor Registry in Japan.
Methods: This study included 316 patients diagnosed with synovial sarcoma between 2006 and 2012. Oncologic outcomes were analyzed using a Cox-hazard regression model. Moreover, the effects of perioperative chemotherapy on outcomes were evaluated using a matched-pair analysis. The oncologic outcomes of patients who did or did not receive chemotherapy were compared (cx + and cx-).
Results: Multivariate analysis revealed significant correlations of age (over 40, hazard ratio [HR] = 0.61, p = 0.043), margin status (marginal resection, HR = 0.18, p < 0.001 and intralesional resection, HR = 0.30, p = 0.013 versus wide resection) with overall survival; surgical margin type (marginal resection, HR = 0.14, p = 0.001 and intralesional resection, HR = 0.09, p = 0.035 versus wide resection) with local recurrence; and postoperative local recurrence (HR = 0.30, p = 0.027) and surgical margin (marginal resection, HR = 0.31, p = 0.023 versus wide resection) with distant relapse-free survival. Before propensity score matching, perioperative chemotherapy was mainly administered for young patients and patients with deeper tumor locations, larger tumors, more advanced-stage disease, and trunk location. The 3-year overall survival, local control, and distant relapse-free survival rates were 79.8%/89.3% (HR = 0.64, p = 0.114), 89.6%/93.0% (HR = 0.37, p = 0.171) and 71.4%/84.5% (HR = 0.60, p = 0.089) in the cx+/cx- groups, respectively. After propensity score matching, 152 patients were selected such that the patient demographics were nearly identical in both groups. The 3-year overall survival, local control, and distant relapse-free survival rates were 71.5%/86.0% (HR = 0.48, p = 0.055), 92.5%/93.3% (HR = 0.51, p = 0.436) and 68.4%/83.9% (HR = 0.47, p = 0.046) in the cx+/cx- groups, respectively.
Conclusion: This large-sample study indicated that the margin status and postoperative disease control were associated directly or indirectly with improved oncologic outcomes. However, the efficacy of perioperative chemotherapy for survival outcomes in synovial sarcoma patients was not proven in this Japanese database analysis.
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http://dx.doi.org/10.1186/s12885-021-08485-1 | DOI Listing |
Cancers (Basel)
December 2024
Sarcoma Unit, The Royal Marsden Hospital and Institute of Cancer Research, London SW3 6JZ, UK.
There has been noteworthy progress in molecular characterisation and therapeutics in soft tissue sarcomas. Novel agents have gained regulatory approval by the FDA. Examples are the tyrosine kinase inhibitors avapritinib and ripretinib in gastrointestinal stromal tumours (GIST), the immune check point inhibitor atezolizumab in alveolar soft part tissue sarcoma, the γ-secretase inhibitor nirogacestat in desmoid tumours, the NTRK inhibitors larotrectinib and entrectinib in tumours with fusions, the mTOR inhibitor nab-sirolimus in PEComa, and the EZH-2 inhibitor tazemetostat in epithelioid sarcoma.
View Article and Find Full Text PDFMedicine (Baltimore)
January 2025
Department of Bone and Joint Surgery, the First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Rationale: Synovial sarcoma (SS) is a rare and highly malignant soft tissue sarcoma. When SS occurs in atypical locations, it can present significant diagnostic challenges. We report a case of paraspinal SS initially misdiagnosed as spinal tuberculosis, highlighting the diagnostic difficulties and the importance of considering SS in the differential diagnosis.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
December 2024
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Synovial sarcoma is a rare malignant mesenchymal neoplasm primarily affecting adolescents and young adults, and it typically arises from deep soft tissues near large joints. Although commonly found in extremities, it can occur in various anatomic locations. We present a rare case of a 29-year-old man with primary pulmonary synovial sarcoma manifesting as a cystic mass masquerading as a lung hydatid cyst.
View Article and Find Full Text PDFJ Hand Surg Asian Pac Vol
January 2025
Auckland Regional Centre for Plastic, Reconstructive and Hand Surgery, Middlemore Hospital, Auckland, New Zealand.
Synovial chondromatosis is a condition that rarely occurs in the hand and wrist. Recurrence and malignant transformation are two potential complications of the disease. We set out to retrospectively review 35 years of practice within our sarcoma and tertiary hand unit and identify recurrence or malignant transformation.
View Article and Find Full Text PDFOncol Rev
December 2024
Department of Ultrasound, The First Affiliated Hospital of Shenzhen University, Shenzhen Second People's Hospital, Shenzhen, China.
Synovial sarcoma (SS) is a rare and malignant mesenchymal neoplasm. We report a case of a 16-year-old Chinese female diagnosed with biphasic synovial sarcoma. The imaging features, surgical procedures and pathological results of the lesion were described in detail.
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